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16-44 Segment 1: Lupus and the Skin

Lupus results when the immune system turns on the body, producing inflammatory attacks on virtually any organ. A minority of patients have lupus only on the skin, and while this is not life threatening, it can still be psychologically devastating.

Reed Pence: The body’s immune system is working to keep us healthy all the time. White blood cells attack and eliminate bacteria, viruses, and cancer cells long before they can do us any harm. But sometimes the immune system goes haywire, and loses the ability to distinguish between those harmful invaders and the cells of our own body. The result is an autoimmune disease, like multiple sclerosis, rheumatoid arthritis… or lupus.

Dr. Christopher Hansen: Lupus also goes by the longer name lupus erythematosus, and it’s really a multi-system autoimmune disorder that can affect different organ systems in the body where your own immune systemis attacking your cells.

Pence: That’s Dr. Christopher Hansen, assistant professor of dermatology at the University Of Utah.

Hansen: Certain people seem to be born with genetic predisposition for developing lupus as with other autoimmune conditions. And then the right event happens, whether that be an infection that flares the immune system and then things go slightly haywire, and rather than attacking the infection, the virus, the bacteria, the immune system starts to recognize your own cells as being bad or foreign. So essentially it’s just a malfunction of the immune system. Any organ system at least can be affected by systemic lupus, including in the heart, lungs muscular skeletal system, neurologic system, kidneys, renal system, the liver can be affected, the lining around the tissues can be affected, as well as the skin.

Pence: According to the Lupus Foundation of America, about one-and-a-half million Americans have lupus. About 90 percent of them are women, and it most often strikes between age 15 and 44. The disease is classified into two main categories — systemic lupus, which can affect any organ, and cutaneous or discoid lupus, which attacks the skin.

Dr. Victoria Worth: Probably 80 percent of patients [who] have systemic lupus get some type of skin involvement. Very often people will present one particular kind of presentation, whether it be joints and systemic symptoms or more skin predominant symptoms. We have to follow people over time to make sure that the disease doesn’t shift.

Pence: Dr. Victoria Werth is professor of dermatology at the University of Pennsylvania and chief of the dermatology section at the Philadelphia V-A Medical Center.

Werth: And I would say potentially 20 percent or so of patients who have more skin predominant disease eventually can meet the criteria for systemic lupus, but many patients who have skin disease seem to be relatively protected against having serious systemic disease. However, we still follow these patients very carefully to make sure that they don’t get renal disease or other involvement from their lupus. With systemic disease I think sometimes it can be a hard diagnosis to make. There’s not really all these visible clues and it requires getting certain blood tests and putting the various puzzle pieces together to make a diagnosis.

Hansen: I’ll have patients that come in that have biopsy indicating lupus in the skin and then the big initial question is whether or not they have systemic lupus and the important thing to realize is that lupus doesn’t always manifest all at once. So it may affect one organ system for a while and then move somewhere else. But the majority of patients it manifests fairly early on if they are going to have systemic involvement or not.

Pence: Common lupus symptoms include extreme fatigue, headaches, painful joints, sun sensitivity, hair loss and anemia. Hansen says people with systemic lupus often have a rash on their skin first. But Werth adds that it often looks different than in those whose lupus is likely to be confined to the skin. It’s an important distinction. Systemic lupus can be extremely serious.

Hansen: So the immune system is activated and creates inflammation so on the skin that is manifested a certain way with redness and scaling and swelling and itching and pain. Other organ systems, such as the kidney, the inflammation can be very silent in terms of what a patient would feel, but the damage is occurring to the cells there despite of the lack of any symptoms. And certain things, depending on what organ system it’s affecting, the symptoms are very different. So when it affects the lining around the lungs it can cause pain with breathing. When it affects the neurologic system it can cause seizures or psychosis or other things, and even stroke.

Pence: Such an inflammatory attack can be caused by a whole variety of malfunctions in virtually any immune system component. So while two cases of lupus may look the same, they may be caused by completely different means with completely different triggers. However, when it comes to lupus on the skin, once someone has a genetic predisposition, the sun is usually to blame. Most cases of cutaneous or discoid lupus occur where the skin is exposed to the sun — on the cheeks, nose and scalp, the back and chest and the back of the hands.

Hansen: Sun seems to play important role in the pathogenesis of many forms of skin lupus. What happens is that particularly significant exposure induces what we call apoptosis or death of some of the skin cells. When those cells die they may expose some of their internal content to the immune system. In turn, that immune system might be hypersensitive or revved up will then attack the skin in those areas where the sun has hit the skin. So that seems to be really the mechanism where that happens.

Pence: However, Hansen admits that doctors don’t understand everything about the sun as a lupus trigger. Sometimes, sun exposure can be a trigger for flares of systemic lupus.

Hansen: Some forms of skin lupus occur in areas where people may have had sun in the past, but not recent sun. So there may be some recall mechanism that occurs with the immune system as well

Pence: Many patients with discoid lupus delay getting help. They may discount the seriousness of that scaly patch on their skin, or believe that the hair loss they’re experiencing is temporary. But Hansen says it’s important to get those patches looked at right away.

Hansen: Some forms of skin lupus can lead to scarring or pigment change in the skin that’s more permanent. The earlier treatment is initiated the less likely that scarring and pigment changes are to occur. One of the things that can happen in patients with lupus on the scalp is they can get skin involvement that is a little deeper down that can destroy hair follicles and that can be quite distressing to patients. If they have patches of hair loss that are essentially irreversible.

Pence: That makes cutaneous or discoid lupus plenty serious even if it’s not life threatening.

Hansen: Generally the skin involvement is not life threatening, but it can have quite a significant psychological impact on patients because it’s in, as we mentioned, some exposed areas. Those are also areas that are generally visible to other people so the involvement of lupus in the skin tends to be something that patients are quite concerned about because that’s what other people can see, They can’t necessarily see their internal organ involvement, but they can see what’s going on the outside.

Pence: Early treatment can keep scarring to a minimum and reverse some of the effects of lupus. And Werth says the first treatment is to keep the trigger from having any influence.

Werth: First treatments are using sunscreen and in the case of lupus we use a high SPF of 70 or higher because even a little bit of light for some patients can be a problem. There is some clothing that can be used to protect against sunlight as well as using hats. We advise people to go out early in the morning or late at night, but not in the middle of the day during peak hours of UV radiation. Those are our initial suggestions in terms of things that people can actually do that make a big difference. We also have certain topical creams that we can use that modify the immune system that can be helpful for people either as a solitary therapy if it’s a mild disease or as an adjunctive therapy with other treatments. If those treatments are not working then we have to go on to more oral medications.

Pence: Topical steroids, or steroids injected into the area of lesions, are also a common treatment.

Werth: Steroids modulate the immune system. They suppress the immune system locally where it is injected. So in the case of lupus where there is inflammation around hair follicles if one does a serum injection the idea is to try to minimize their inflammation in the skin that may be disrupting hair follicles and causing problems of discoid lupus.

Hansen: For people that have more wide spread skin involvement the mainstay of treatment for 50 years or so has been antimalarial therapy. People are always a little curious why medicines that are used to prevent malaria would help lupus, particularly lupus in the skin. That’s the area that is not fully understood, but we know antimalarial seems to have an effect on some of the sun effects on the skin and as well as actually an effect on the immune system that seems to quiet the activity in the skin and can be quite helpful for the majority of patients with skin lupus.

Pence: If anti-malarial don’t work, and they do in more than half of patients, then doctors turn to immunosuppressant’s. But newer treatments in development might be able to combat lupus without tamping down the immune system.

Dr. Betty Diamond: My own approach to this is to think about less immunosuppressant therapies for lupus; to think about things that we can target that don’t leave people extremely immune-compromised. The third of lupus patients now die of infectious causes where our therapy certainly makes a bad contribution

Pence: That’s Dr. Betty Diamond, a rheumatologist and immunologist who is an investigator at the Feinstein Institute for Medical Research. She says that doctors have had to treat most cases of lupus the same way up ‘til now. Only one drug has ever been developed and approved specifically for lupus. But Diamond says new treatments will differentiate between the different malfunctions in the immune system that cause lupus.

Diamond: I think that we’re starting to get some clues from genetics and what we need to do is learn whether people whose genetic risks involve particular genes. The different treatments over time in people whose risk for disease involves different genes. My own feeling is that when you have a rippling flare of disease activity it will probably be that you can treat everybody the same way to dampen the immune activation. But to maintain somebody in clinical remission we may need to have therapies that are based on your inciting genetic abnormality.

Pence: Once lupus strikes, it lasts a lifetime. There is no cure. But experts say it can be successfully managed in the vast majority of cases. The key is getting treatment early, before lupus has the chance to inflict irreversible damage.

Pence: You can find out much more about lupus from the Lupus Foundation of America at lupus.org. You can find out more about all of our guests on our website, radiohealthjournal.net.